0000000000361905
AUTHOR
Kebede Hussein
Transfusion-dependency at presentation and its acquisition in the first year of diagnosis are both equally detrimental for survival in primary myelofibrosis - prognostic relevance is independent of IPSS or karyotype
The International Prognostic Scoring System (IPSS) and karyotype are useful tools for risk stratification in primary myelofibrosis (PMF). We examined the additional prognostic impact of red blood cell transfusion need among 254 consecutive patients (median age, 59 years). Sixty-two patients ( approximately 24%) required transfusions at diagnosis whereas 22 ( approximately 9%) became transfusion-dependent and 170 remained transfusion-independent during the first year postdiagnosis; after a median follow-up of 55 months, the respective median survivals were 35, 25, and 117 months (P < 0.01). Multivariable analysis confirmed the IPSS- and karyotype-independent prognostic weight of transfusion …
Transfusion Need at Diagnosis or Its Development During the First Year of Diagnosis in Primary Myelofibrosis: Effect On Survival and Correlation with JAK2 and TET2 Mutational Status.
Abstract Abstract 1909 Poster Board I-932 Background: The International Prognostic Scoring System (IPSS) for primary myelofibrosis (PMF) utilizes five independent predictors of inferior survival; of these, a hemoglobin level <10 g/dL has the highest impact on survival (Cervantes et al. Blood 2009;113:2895). In the current study, we examined the additional prognostic impact of transfusion need at diagnosis or becoming transfusion-dependent in the first year of diagnosis. These events were also correlated with JAK2 or TET2 mutational status. Methods: Patients were selected from the Mayo Clinic PMF database based on availability of bone marrow histology and IPSS-relevant information at diag…
Mature Survival Data for 176 Patients Younger Than 60 Years With Primary Myelofibrosis Diagnosed Between 1976 and 2005: Evidence for Survival Gains in Recent Years
In the past 20 years, management of primary myelofibrosis (PMF) has incorporated new treatment approaches, but survival benefits have not been confirmed in controlled studies. This retrospective study includes 176 consecutive patients younger than age 60 years in whom PMF was diagnosed during a 30-year period (1976-2005). Median age at diagnosis was 50 years (range, 18-59 years), and 98 patients (55%) were men. At the time of this report, 99 patients (56%) had died; the 77 surviving patients were followed up for a median of 8 years (range, 4-24 years). Overall median survival was 9.2 years, and 15- and 20-year survival rates were 32% and 20%, respectively. According to the Dupriez Prognosti…
Chromosome 1 abnormalities in myeloid malignancies: a literature survey and karyotype-phenotype associations.
Chromosome 1 is the largest human chromosome and contains over 1600 known genes and 1000 novel coding sequences or transcripts. It is, therefore, not surprising that recurrent chromosome 1 abnormalities are regularly encountered in both neoplastic and non-neoplastic medical conditions. The current review is focused on myeloid malignancies where we summarize the relevant published literature and discuss specific karyotype-phenotype associations. We show that chromosome 1 abnormalities are most frequent in BCR-ABL-negative classic myeloproliferative neoplasms (MPN): polycythemia vera (PV), essential thrombocythemia (ET), and primary myelofibrosis (PMF). Specific abnormalities include duplicat…